Document Detail

Subcorneal pustular dermatosis in childhood: a case report and review of the literature.
Jump to Full Text
MedLine Citation:
PMID:  23365765     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) is a rare, benign, chronic, sterile pustular eruption which usually develops in middle-age or elderly women; it is rarely seen in childhood and adolescence. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid blisters. Histologically the most important feature is a subcorneal accumulation of neutrophils with the absence of spongiosis or acantholysis, although acantholysis may be reported in older lesions. In this paper we present the case of a 7-year-old boy diagnosed with SCPD based on the characteristic clinical and histological features. Dapsone has been successfully used in the treatment of the disease.
Massimiliano Scalvenzi; Franco Palmisano; Maria Carmela Annunziata; Ernesto Mezza; Immacolata Cozzolino; Claudia Costa
Related Documents :
24021365 - Shiitake mushroom-induced flagellate erythema: a striking case and review of the litera...
23422635 - An erupted odontoma associated with pigmentation: a histogenetic and histological persp...
18457705 - Veterinary endodontics: an online study guide.
23365765 - Subcorneal pustular dermatosis in childhood: a case report and review of the literature.
6698535 - Neuropathology of familial erythrophagocytic lymphohistiocytosis: six cases and review ...
945725 - Hemangioblastoma of the spinal cord. review and report of five cases.
Publication Detail:
Type:  Journal Article     Date:  2013-01-09
Journal Detail:
Title:  Case reports in dermatological medicine     Volume:  2013     ISSN:  2090-6463     ISO Abbreviation:  Case Rep Dermatol Med     Publication Date:  2013  
Date Detail:
Created Date:  2013-01-31     Completed Date:  2013-02-01     Revised Date:  2013-04-18    
Medline Journal Info:
Nlm Unique ID:  101591808     Medline TA:  Case Rep Dermatol Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  424797     Citation Subset:  -    
Department of Dermatology, Federico II University, Via Pansini 5, 80131 Naples, Italy.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Case Rep Dermatol Med
Journal ID (iso-abbrev): Case Rep Dermatol Med
Journal ID (publisher-id): CRIM.DM
ISSN: 2090-6463
ISSN: 2090-6471
Publisher: Hindawi Publishing Corporation
Article Information
Download PDF
Copyright © 2013 Massimiliano Scalvenzi et al.
Received Day: 28 Month: 11 Year: 2012
Accepted Day: 27 Month: 12 Year: 2012
Print publication date: Year: 2013
Electronic publication date: Day: 9 Month: 1 Year: 2013
Volume: 2013E-location ID: 424797
PubMed Id: 23365765
ID: 3556408
DOI: 10.1155/2013/424797

Subcorneal Pustular Dermatosis in Childhood: A Case Report and Review of the Literature
Massimiliano Scalvenzi1*
Franco Palmisano1
Maria Carmela Annunziata1
Ernesto Mezza2
Immacolata Cozzolino2
Claudia Costa1
1Department of Dermatology, Federico II University, Via Pansini 5, 80131 Naples, Italy
2Department of Biomorphological and Functional Sciences, Federico II University, 80131 Naples, Italy
Correspondence: *Massimiliano Scalvenzi:
[other] Academic Editors: B. Kumar and J. Y. Lee

1. Introduction

Subcorneal pustular dermatosis (SCPD, Sneddon-Wilkinson disease) is a rare chronic, relapsing, pustular eruption that was first described by Sneddon and Wilkinson in [1]. It has been reported more frequently in women in the age group 40–50 years, however this disease can occur more rarely during the childhood [2]. Patients characteristically have a history of a relapsing symmetrical sterile pustular eruption involving the trunk, intertriginous areas, and flexor aspects of the limbs. The face, palms, soles, and mucous membranes are usually spared. The primary lesion is a small pustule arising on normal skin or slightly erythematous base. The pustules classically are described as half-pustular, half-clear fluid blisters that coalesce to form annular or serpiginous patterns. They are flaccid and rupture easily, resulting in superficial scaling, crusting, and faint hyperpigmentation. The condition is benign unless associated with underlying malignancy, which worsens the prognosis [3].

Its exact pathophysiology is unknown and its exact nosological classification is still controversial. The salient histological feature is a subcorneal accumulation of neutrophils with the absence of spongiosis or acantholysis.

Therapeutically, Dapsone is the first-line treatment in SPD [4, 5], and other treatment options are etretinate, acitretin, PUVA, narrow-band (TL-1) UVB phototherapy, and colchicine [6, 7].

2. Case Report

A 7-year-old boy was admitted to our clinic with a 3-week-old itchy eruption located on the trunk, on the limbs, and on the face. He had a history of atopic dermatitis, while his familiar anamnesis was negligible. A complete blood count and the studies of serum biochemistry showed normal results; moreover serum protein electrophoresis had negative results. The lesions initially developed on the trunk and upper extremities, then they progressed up to involve almost the whole body surface. The palms, soles, and mucous membrane were spared, and no lymphadenopathy or hepato-splenomegaly was present. There were no abnormalities of the nails and tongue.

The dermatologic examination revealed multiple-grouped flaccid pustules varying in size from 2 to 10 mm that tended to coalesce to form annular, circinate, or serpiginous pattern and superficial crusts on the normal or mildly erythematous skin, of the face, trunk, and extremities (Figure 1). Healed lesions presented residual hyperpigmentation and new lesions in the periphery.

Taking into consideration a suspected diagnosis of SCPD, the patient was treated with oral antihistamines and with a topic solution of eosin (2%); moreover an incisional biopsy of a lesion on the sternal region was carried out.

Histopathology demonstrated a subcorneal vesiculo-bullous dermatitis (Figure 2); the pustule is located immediately below the stratum corneum and contains mainly neutrophils with few eosinophils. The underlying epidermis to the pustule show slight intercellular edema. In the dermis, superficial blood vessels are surrounded by a nonspecific mixed inflammatory cell infiltrate consisting of neutrophils and mononuclear cells. Direct immunofluorescence studies are negative for immunoglobulin A (IgA) intercellular accumulation (Figure 3). On the base of this finding, associated to histopathological features and the clinical date, a diagnosis of subcorneal pustular dermatitis (SCPD, Sneddon-Wilkinson disease) was made.

The patient was treated with 30 mg of oral Diaminodiphenylsulfone (Dapsone, 1 mg/kg/day). The cutaneous lesions were almost completely healed at the first followup, within 2 weeks (Figure 4). After 4 weeks, treatment with Dapsone was continued on alternate days for another month, at the same daily dosage.

After 3 months, the patient is still monitored for the followup at our hospital every 2 weeks.

3. Discussion and Review

Children can have various bullous and pustular skin diseases like pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, pustular bacterid, and psoriasis as well as dermatitis herpetiformis [8, 9]; all of these were once thought to be unique to people in the fourth-fifth decade of life. Subcorneal pustular dermatosis appears to be another one of these diseases.

The etiology of the disease is still obscure. There are well-documented SCPD in associations with benign monoclonal IgA gammopathy [10] and pyoderma gangrenosum [11]. There are also reports in association with IgA myeloma [3], SAPHO syndrome [12], Crohn's disease [13], Sjogren's syndrome [14], rheumatoid arthritis [15], and thyroidal diseases [16].

In our case, the history, physical examination, and laboratory results did not reveal any systemic associations. Moreover some cases, which were consistent with SCPD according to the clinical and histologic features, have been reported with the presence of an intercellular IgA deposition within the epidermis [17].

This disease involves more frequently the trunk, intertriginous areas, and flexor aspects of the limbs; more rarely the face is implicated, as in this case. Pustules on palms and soles have also been reported [18], while mucous membranes are almost never affected.

The differential diagnosis of SCPD includes pustular psoriasis, impetigo, dermatophyte infection, and immunobullous diseases (dermatitis herpetiformis, pemphigus, linear IgA disease, and intercellular IgA diseases). Unlike pustular psoriasis, nails and scalp are uncommonly affected in SCPD; moreover spongiform pustules, formation of microabscess, and elongation of rete ridges do not occur in classical Sneddon-Wilkinson disease [17]. In generalized pustular psoriasis, patient cases to have fever and leukocytosis [19]. A dermatophyte infection can be easily excluded with a direct microscopic examination of fungal elements. The differential diagnosis from impetigo may be difficult; the possible bacterial contamination, not always, can be demonstrated by Gram stain. IgA deposition in the dermal papillae distinguishes SCPD from dermatitis herpetiformis.

In the IgA pemphigus subtype, generally, the acantholysis tends to be more pronounced than in SCPD, Sneddon-Wilkinson disease, in this regard, in our sample, were not observed acantholytic cells; moreover, in the IgA pemphigus, DIF studies demonstrate intercellular IgA accumulation in squamous cells.

In Sneddon and Wilkinson's original report [1], the average age of the patients was 54.8 years.

Only 15 cases of pediatric SCPD are described in literature [2, 2029].

Sarkany [20] had a patient 10 years of age and Beck et al. [21] had had a 29-years-old patient whose disease had started when the patient was 3 months old. Baker and Ryan [22, 23], in their two reviews of 104 cases and 155 cases of pustular psoriasis, reported that generalized pustular psoriasis had its onset before the age of 11 in only four children (three boys and one girl). All of the four children had the “annular pattern” of pustular psoriasis that according to the authors, somewhat resembled subcorneal pustular dermatosis.

R. E. Burns, MD, had a patient with subcorneal pustular dermatosis who gave birth to a child with similar skin lesions that lasted seven days (oral communication, April 1973) [2].

Desmons and Defrenne [24] also described in 1973 a case of a 12-year-old boy with a recurrent subcorneal pustular dermatosis.

Johnson and Cripps [2] report two 3-year-old children, a boy and girl, with Sneddon-Wilkinson syndrome; in both of these children some of the flares of the disease followed infections.

Garg et al. [25] reported in 1985 a case of a young boy affected by SCPD successfully treated with dapsone.

In 1986 Rosińska-Borkowska and Henig published a case report about a 30-month-old patient with SCPD [26]; in the same year Park et al. described a 12-year-old boy with a 5-year history of recurrent generalized dermatoses with scales, crusts, and pustole: Dapsone and prednisolone with a topical fluocinolone acetonide did not produce improvement [27]. The skin lesions cleared completely after 11 exposures of UV-B three times a week. After 8 months of followup, there has been no recurrence.

In 2003 Koçak et al. [28] had a 13-year-old girl with SCPD in terapy with Dapsone. After 3 weeks of treatment, the lesions regressed almost completely, but her hemoglobin decreased from 12.5 mg/dL to 9.6 mg/dL; for this reason systemic treatment was stopped and topical steroid ointment was initiated with acceptable clinical results.

The last case reported in literature shows a case of juvenile subcorneal pustular dermatosis successfully treated with acitretin [29].

Even if SCPD is an uncommon condition in childhood, it must be considered as a possible cause of sterile pustular eruptions in a child. An accurate physical examination, a complete blood count, and studies of serum biochemistry are strongly recommended to exclude a pathology in association. Dapsone remains the treatment of choice but its safe is still debatable and a close followup is required.

Conflict of Interests

The authors declared that they have no conflict of interests.

1. Sneddon IB,Wilkinson DS. Subcorneal pustular dermatosisThe British Journal of DermatologyYear: 195668123853942-s2.0-000137919013396129
2. Johnson SA,Cripps DJ. Subcorneal pustular dermatosis in childrenArchives of DermatologyYear: 1974109173772-s2.0-00159979384588204
3. Atukorala DN,Joshi RK,Abanmi A,Jeha MT. Subcorneal pustular dermatosis and IgA myelomaDermatologyYear: 199318721241262-s2.0-00272205148358100
4. Hönigsmann H,Trautinger F,Wolff K. Freedberg IM,Eisen AZ,Wolff KSubcorneal pustular dermatosis (Sneddon-Wilkinson disease)Dermatology in General MedicineYear: 19995th editionNew York, NY, USAMcGraw-Hill654666
5. Hogan PA. Schachner LA,Hansen RCPapulosquamous diseasePediatric DermatologyYear: 20033rd editionEdinburgh, UKMosby643681
6. Todd DJ,Bingham EA,Walsh M,Burrows D. Subcorneal pustular dermatosis and IgA paraproteinaemia: response to both etretinate and PUVABritish Journal of DermatologyYear: 199112543873892-s2.0-00260014131954130
7. Marlière V,Beylot-Barry M,Beylot C,Doutre MS. Successful treatment of subcorneal pustular dermatosis (Sneddon-Wilkinson disease) by acitretin: report of a caseDermatologyYear: 199919921531552-s2.0-1714444318710559583
8. Mintz EM,Morel KD. Clinical features, diagnosis, and pathogenesis of chronic bullous disease of childhoodDermatologic ClinicsYear: 201129345946221605812
9. Monteagudo Paz AF,Betlloch Mas I,Latorre Martínez N. Chronic bullous dermatosis of childhoodAnales de PediatríaYear: 2011754277278
10. Kasha EE,Epinette WW. Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) in association with a monoclonal IgA gammopathy: a report and review of the literatureJournal of the American Academy of DermatologyYear: 19881958548582-s2.0-00237987263056995
11. Scerri L,Zaki I,Allen BR. Pyoderma gangrenosum and subcorneal pustular dermatosis, without monoclonal gammopathyBritish Journal of DermatologyYear: 199413033983992-s2.0-00282352688148286
12. Scarpa R,Lubrano E,Cozzi R,Ames PR,Oriente CB,Oriente P. Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome): another cutaneous manifestation of SAPHO syndrome?British Journal of RheumatologyYear: 19973656026032-s2.0-00311351059189065
13. Delaport E,Colombel JF,Nguyen-Malifer C,et al. Subcorneal pustular dermatosis in a patient with Crohn’s diseaseActa Dermato-VenereologicaYear: 1992723013021357895
14. Tsuruta D,Matsumura-Oura A,Ishii M. Subcorneal pustular dermatosis and Sjögren’s syndromeInternational Journal of DermatologyYear: 200544119559572-s2.0-2844449941016336533
15. Butt A,Burge SM. Sneddon-Wilkinson disease in association with rheumatoid arthritisBritish Journal of DermatologyYear: 199513223133152-s2.0-00289131577888376
16. Taniguchi S,Tsuruta D,Kutsuna H,Hamada T. Subcorneal pustular dermatosis in a patient with hyperthyroidismDermatologyYear: 1995190164662-s2.0-00288401727894101
17. Hashimoto T,Inamoto N,Nakamura K,Nishikawa T. Intercellular IgA dermatosis with clinical features of subcorneal pustular dermatosisArchives of DermatologyYear: 19871238106210652-s2.0-00231899923307637
18. Takematsu H,Tagami H. Quantification of chemotactic peptides (C5a anaphylatoxin and IL-8) in psoriatic lesional skinArchives of DermatologyYear: 1993129174802-s2.0-00274719718420495
19. Braun-Falco O,Plewig G,Wolf HH,et al. Pustular DiseasesYear: 2000Milan, ItalySpringer
20. Sarkany I. Subcorneal pustular dermatosisBritish Journal of DermatologyYear: 195870p. 307
21. Beck AL,Kipping HL,Crissey JT. Subcorneal pustular dermatosis. Report of a caseArchives of DermatologyYear: 1961836276292-s2.0-7865111552713688367
22. Baker H,Ryan TJ. Generalized pustular psoriasis. A clinical and epidemiological study of 104 casesBritish Journal of DermatologyYear: 196880127717932-s2.0-00143756454236712
23. Ryan TJ,Baker H. The prognosis of generalized pustular psoriasisBritish Journal of DermatologyYear: 19718554074112-s2.0-00151522125132156
24. Desmons F,Defrenne C. Recurrent subcorneal pustular dermatitis in a boy of 12 years. Diagnosis and etiologyArchives Belges De DermatologieYear: 19732943133152-s2.0-00157564334802307
25. Garg BR,Sait MA,Baruah MC. Subcorneal pustular dermatosis in a young boyIndian Journal of DermatologyYear: 198530421232-s2.0-00221322523843557
26. Rosińska-Borkowska D,Henig E. Sneddon-Wilkinson disease in a 30-month-old childPrzeglad DermatologicznyYear: 19867354064082-s2.0-00227715763296010
27. Park YK,Park HY,Bang DS,Cho CK. Subcorneal pustular dermatosis treated with phototherapyInternational Journal of DermatologyYear: 19862521241262-s2.0-00226343993516894
28. Koçak M,Birol A,Erkek E,et al. Juvenile subcorneal pustular dermatosis: a case reportPediatric DermatologyYear: 200320157592-s2.0-003753129712558849
29. Yayli S,Bahadir S,Alpay K,Çimşit G,Reis A. A case of juvenile subcorneal pustular dermatosis successfully treated with acitretinInternational Journal of DermatologyYear: 2006459113111332-s2.0-3374828001916961541

Article Categories:
  • Case Report

Previous Document:  "Segmental necrotizing granulomatous neuritis": a rare manifestation of hansen disease-report of 2 c...
Next Document:  Arms lift in a case of pseudoxanthoma elasticum.